alpha-L-Iduronidase/IDUA Antibody [Biotin]

• Applications: WB
• Clonality: Polyclonal
• Host: Sheep
• Conjugate: Biotin
• Concentration: LYOPH

alpha-L-Iduronidase/IDUA Antibody [Biotin] Summary

• Immunogen: Mouse myeloma cell line NS0-derived recombinant human alpha ‑L‑Iduronidase/IDUA
  Ala26-Pro653 (Ala26Thr)
  Accession # AAA81589
• Specificity: Detects human alpha ‑L‑Iduronidase/IDUA in Western blots.
• Source: N/A
• Isotype: IgG
• Clonality: Polyclonal
• Host: Sheep
• Gene: IDUA
• Purity Statement: Antigen Affinity-purified

Packaging, Storage & Formulations

• Storage: Use a manual defrost freezer and avoid repeated freeze-thaw cycles.
  • 12 months from date of receipt, -20 to -70 °C as supplied.
  • 1 month, 2 to 8 °C under sterile conditions after reconstitution.
  • 6 months, -20 to -70 °C under sterile conditions after reconstitution.
• Buffer: Lyophilized from a 0.2 μm filtered solution in PBS with BSA as a carrier protein.
• Preservative: No Preservative
• Concentration: LYOPH
• Reconstitution Instructions: Reconstitute at 0.2 mg/mL in sterile PBS.

Notes

This product is produced by and ships from R&D Systems, Inc., a Bio-Techne brand.

Background

alpha -L-Iduronidase encoded by the IDUA gene is an important enzyme required for the lysosomal degradation of glycosaminoglycans (GAGs). It hydrolyzes the non‑reducing terminal alpha -L-iduronic acid residues in GAGs including dermatan sulfate and heparan sulfate. Mutations in IDUA that result in enzymatic deficiency lead to the autosomal recessive disease mucopolysaccharidosis type I (MPS I) (1). MPS I causes progressive cellular, tissue and organ damage, and several clinical studies using enzyme replacement therapy have shown promising benefits (2).

1. Scott, H.S. et al. (1995) Hum. Mutat. 6:288.
2. Wraith, J.E. (2005) Expert Opin. Pharmacother. 6:489.

Limitations

This product is for research use only and is not approved for use in humans or in clinical diagnosis. Primary Antibodies are guaranteed for 1 year from date of receipt.

Bioinformatics

• Gene Symbol: IDUA